Publications by authors named "Sharon Webster"

Article Synopsis
  • Activated phosphoinositide 3-kinase delta (PI3Kδ) syndrome (APDS) is a rare genetic disease that disrupts the immune system, causing various symptoms that usually begin in childhood.
  • In a phase III clinical trial, a drug called leniolisib was shown to effectively reduce lymph node swelling and increase naïve B cell levels in both adolescents and adults with APDS compared to those given a placebo.
  • The study found that leniolisib was well-tolerated across age groups and suggests it could be a promising treatment option for managing APDS by addressing the underlying cause rather than just the symptoms.
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Article Synopsis
  • Activated phosphoinositide 3-kinase delta (PI3Kδ) syndrome (APDS) is a genetic immune disorder that leads to frequent infections and abnormal immune cell growth, but treatment with leniolisib, a selective PI3Kδ inhibitor, has shown promising results.
  • In a 6-year follow-up study of 6 adult patients who received leniolisib, improvements were noted in health-related quality of life (HRQoL), with most patients reporting better physical capabilities and reduced medication usage.
  • Overall immune responses improved, with significant reductions in certain immune cell types and fewer infections, supporting leniolisib as a viable long-term treatment for APDS, while adverse events were mostly mild.
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Article Synopsis
  • Activated phosphoinositide 3-kinase delta (PI3Kδ) syndrome (APDS) is a genetic condition characterized by overactive PI3Kδ, leading to immune issues like recurrent infections and autoimmunity.
  • Leniolisib, a targeted inhibitor of PI3Kδ, showed promise in improving immune function and reducing complications like lymphoproliferation in patients with APDS over a 12-week period.
  • An ongoing study involving 37 patients showed that most experienced mild to moderate side effects, but leniolisib significantly decreased infection rates and improved overall health, with many patients needing less immunoglobulin therapy.
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Article Synopsis
  • Activated phosphoinositide 3-kinase delta syndrome (APDS) is an immune disorder that leads to various health issues like infections and autoimmunity due to overactive PI3Kδ signaling.
  • A phase 3 clinical trial tested leniolisib, a specific inhibitor of PI3Kδ, on 31 patients aged 12 and older, comparing its effects against a placebo over 12 weeks.
  • Results showed that leniolisib significantly reduced lymph node size and increased naïve B cells, indicating improved immune function, while also being well-tolerated with fewer adverse events compared to placebo.
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Objectives: Recent data have demonstrated benefits of pharmacist-led protocols for chronic disease state management in the primary care setting. Health coaching has also been shown to improve patient outcomes and reduce health care costs. A program was initiated in August 2017 at a rural, free clinic to provide team-based, patient-centered care management through the use of pharmacist-provider collaborative practice and health coaching for patients with chronic diseases such as diabetes, hypertension, and hyperlipidemia.

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Pathogenic gain-of-function variants in the genes encoding phosphoinositide 3-kinase δ (PI3Kδ) lead to accumulation of transitional B cells and senescent T cells, lymphadenopathy, and immune deficiency (activated PI3Kδ syndrome [APDS]). Knowing the genetic etiology of APDS afforded us the opportunity to explore PI3Kδ inhibition as a precision-medicine therapy. Here, we report in vitro and in vivo effects of inhibiting PI3Kδ in APDS.

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Rationale: Patients with autoimmune lymphoproliferative syndrome (ALPS), a disorder of impaired lymphocyte apoptosis, often undergo radiographic chest imaging to evaluate the presence and progression of lymphadenopathy. These images often lead to parenchymal and interstitial lung findings of unclear clinical significance.

Objectives: To characterize the pulmonary findings associated with ALPS and to determine if lung abnormalities present on computed tomographic (CT) imaging of the chest correlate with infection or functional status.

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